Copper metabolism after living related liver transplantation for Wilson's disease.
Xuehao Wang,Feng Cheng,Feng Zhang,Xiangcheng Li,Jian-Ming Qian,Lianbao Kong,Hao Zhang,Guoqiang Li +7 more
TLDR
In this paper, living-related liver transplantation (LRLT) for Wilson's disease (WD) patients with the fulminant form and end-stage liver failure was discussed.Citations
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Long term results of liver transplantation for Wilson’s disease: Experience in France
Olivier Guillaud,Jérôme Dumortier,Rodolphe Sobesky,Dominique Debray,Philippe Wolf,Claire Vanlemmens,François Durand,Yvon Calmus,Christophe Duvoux,Sébastien Dharancy,Nassim Kamar,Karim Boudjema,Pierre Bernard,Georges-Philippe Pageaux,Ephrem Salamé,Jean Gugenheim,Alain Lachaux,Dalila Habes,Sylvie Radenne,Jean Hardwigsen,Olivier Chazouillères,Jean-Marc Trocello,Philippe Ichai,Sophie Branchereau,Olivier Soubrane,Denis Castaing,Emmanuel Jacquemin,Didier Samuel,Jean-Charles Duclos-Vallée +28 more
TL;DR: Liver failure associated with WD is a rare indication for LT (<1%), which achieves an excellent long-term outcome, including renal function.
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Liver transplantation for Wilson's disease.
TL;DR: Liver transplantation restores normal biliary copper excretion and promotes removal of copper from extrahepatic sites and prevents disease recurrence in Wilsons's disease patients.
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Living-related liver transplantation for Wilson's disease.
Sumihito Tamura,Yasuhiko Sugawara,Yoji Kishi,Nobuhisa Akamatsu,Junichi Kaneko,Masatoshi Makuuchi +5 more
TL;DR: For advanced liver failure in WD, LRLT is considered a valuable life‐saving option and the improvement of neurologic symptoms, however, requires further evaluation.
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Influence of Liver Transplantation on Neuropsychiatric Manifestations of Wilson Disease.
Mehmet Ali Yagci,Ali Tardu,Servet Karagul,Ismail Ertugrul,Volkan Ince,Serdar Kirmizi,Bülent Ünal,Burak Isik,Cuneyt Kayaalp,Sezai Yilmaz +9 more
TL;DR: Liver transplantation for Wilson disease can provide some improvement of the neuropsychological symptoms in addition to the hepatic recovery, and this study found this to be the case with nine of 42 Wilson disease patients.
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Neurological manifestations in Wilson’s disease –possible treatment options for symptoms
TL;DR: Wilson’s disease is a neurodegenerative disorder that presents mainly with liver symptoms, but may be accompanied by various neurological manifestations and drugs used to treat movement disorders such as tremor, parkinsonism, or dystonia may also alleviate these symptoms in patients with WD.
References
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The Wilson disease gene is a copper transporting ATPase with homology to the Menkes disease gene.
Rudolph E. Tanzi,Konstantin Petrukhin,Igor P. Chernov,Jean-Luc Pellequer,Wilma Wasco,B Ross,Donna M. Romano,Enrico Parano,Lorenzo Pavone,Linda M. Brzustowicz +9 more
TL;DR: The predicted functional properties of the pWD gene together with its strong homology to Mc1, genetic mapping data and identification of four independent disease–specific mutations, provide convincing evidence that pWD is the Wilson disease gene.
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Successful Liver Transplantation from a Living Donor to Her Son
Russell W. Strong,Stephen V. Lynch,T. H. Ong,Hidetoshi Matsunami,Yuichi Koido,Glenda A. Balderson +5 more
TL;DR: This was the first experience with the orthotopic reduced-size liver transplantation technique, which involved the retention of the recipient's inferior vena cava, and the principle of transplanting a portion of the liver from an adult into a child has been accepted in many centers.
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Isolation and characterization of a human liver cDNA as a candidate gene for Wilson disease.
TL;DR: The putative copper and ATP-binding domains of the human Menkes disease gene were used as probes to screen a human liver cDNA library at reduced stringency and suggest that this cDNA is a candidate gene for Wilson disease and that the protein encoded at this locus is a member of the P-type ATPase family.
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Mapping, Cloning and Genetic Characterization of the Region Containing the Wilson Disease Gene
Konstantin Petrukhin,Stuart G. Fischer,Mario Pirastu,Rudolph E. Tanzi,Igor P. Chernov,Marcella Devoto,Linda M. Brzustowicz,Eftihia Cayanis,Emilia Vitale,James J. Russo +9 more
TL;DR: This work developed yeast artificial chromosome and cosmid contigs, and microsatellite markers which span the WD gene region, and predicts that approximately half of all WD mutations will be rare in the American and Russian populations.
Journal ArticleDOI
Wilson's disease in patients presenting with liver disease: A diagnostic challenge
P. Steindl,Peter Ferenci,H P Dienes,Georg Grimm,Ingrid Pabinger,Christian Madl,Theresia Maier-Dobersberger,A. M. Herneth,Brigitte Dragosics,Siegfried Meryn,P. Knoflach,G Granditsch,Alfred Gangl +12 more
TL;DR: The commonly used clinical and laboratory parameters are not sufficient to exclude the diagnosis of Wilson's disease in patients with liver disease of unknown origin.